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两兄弟胱氨酸病的延迟诊断:肾脏组织学和超微结构研究

[Late diagnosis of cystinosis in 2 brothers: histological and ultrastructural renal study].

作者信息

Blanc-Brunat N, Berthoux F, Colon S, Janin G

出版信息

Arch Fr Pediatr. 1978 May;35(5):486-503.

PMID:678028
Abstract

Cystinosis was discovered by chance in two adolescent boys who had proteinuria with minor tubular abnormalities. Renal biopsies were examined by light microscopy, electron microscopy and immunofluorescence. There were few histological changes but crystals were present in the epithelial cells of the glomerulus and occasionally in the tubules. A further unusual feature was the detection of IgA deposits in the mesangium.

摘要

胱氨酸病偶然发现于两名患有蛋白尿且伴有轻微肾小管异常的青春期男孩。对肾活检组织进行了光镜、电镜和免疫荧光检查。组织学变化很少,但在肾小球上皮细胞中存在晶体,偶尔在肾小管中也有。另一个不寻常的特征是在系膜中检测到IgA沉积物。

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